Survival and Predictors of Mortality in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry
Survival and Predictors of Mortality in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry
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DOI：
10.1002/acr.22121
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发表时间：
2014-03-01
影响因子：
4.7
通讯作者：
Steen, Virginia D.
中科院分区：
文献类型：
--
作者：
Chung, Lorinda;Domsic, Robyn T.;Steen, Virginia D.
目的评估在美国 SSc 中心进行常规 PAH 筛查的系统性硬化症 (SSc) 相关肺动脉高压 (PAH) 患者的累积生存率并确定死亡率的独立预测因子。方法硬皮病登记中的肺动脉高压评估和结果识别是 PAH 高风险或明确的 SSc 患者的前瞻性登记 入组后 6 个月内通过右侧心导管检查诊断出肺动脉高压。这些分析仅纳入世界卫生组织 I 组 PAH 患者（平均肺动脉压 25 mm Hg，肺毛细血管楔压 15 mm Hg，无明显间质性肺疾病）。 结果 总共，对 131 名发生 PAH 的 SSc 患者进行了平均 SD 为 2.0 +/- 1.4 年的随访。 1年、2年和3年累积生存率分别为93%、88%和75%。多变量分析显示，年龄 >60 岁（风险比 [HR] 3.0，95% 置信区间 [95% CI] 1.1-8.4）、男性（HR 3.9，95% CI 1.1-13.9）、功能等级 (FC) IV 状态（HR 6.5，95% CI 1.8-22.8）和一氧化碳扩散能力 (DLco)
ObjectiveTo assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US.MethodsThe Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure 25 mm Hg and pulmonary capillary wedge pressure 15 mm Hg without significant interstitial lung disease) were included in these analyses.ResultsIn total, 131 SSc patients with incident PAH were followed for a mean SD of 2.0 +/- 1.4 years. The 1-, 2-, and 3-year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age >60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1-8.4), male sex (HR 3.9, 95% CI 1.1-13.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8-22.8), and diffusing capacity for carbon monoxide (DLco)